Sclerema Neonatorum Research Articles

Urea and electrolyte levels in the serum in sclerema neonatorum

The urea and serum electrolyte concentrations were measured in 35 infants with mild to severe sclerema neonatorum. These values were compared with those obtained in a control group of 34 healthy infants matched for age and weight. Significant changes were found in the sera of the infants with severe sclerema. They had markedly raised urea and potassium levels, while the CO₂ content was frequently depressed. These findings, furthermore, were determined to be of prognostic significance. A serum potassium level greater than 6.0 mEq. per liter and/or a CO₂ combining power below 15.0 mEq. per liter were often associated with a fatal outcome.

Sclerema Neonatorum

Sclerema Neonatorum

Sclerema neonatorum--a sign, not a disease.

Experience indicates that sclerema neonatorum is a morbid sign which occurs during the course of a potentially fatal disease process. In a study of 18 infants who exhibited the sign, 14 were found to have pneumonia. Sclerema developed as a terminal event (within 8 hours of death) in five instances. In six others, the time of appearance ranged from 14 hours to 5 days before death. In these cases death was the result of a progressive disease. Of the seven remaining infants, four were treated with steroids and three, without. In these cases, sclerema disappeared as the infants recovered from the underlying disease. Rapid application of therapy specific for the underlying disease is necessary and sufficient to reverse the sclerema. Cortisone is not essential to treatment.

NEONATAL SKIN DISORDERS

To the Editor : The article Adipose Derangements of the Newborn is another attempt to clarify the confusion regarding three distinct neonatal skin disorders. The use of the term sclerema neonatorum for any one of them is unfortunate since all three have, at one time or another, been labeled with that term. Subcutaneous fat necrosis is the most easily differentiated, especially in the localized form. Preagonal induration is more descriptive of the clinical state of the infants with the condition that the author has chosen to label sclerema neonatorum. The case report under the heading of scleredema is an example of the third neonatal skin disorder, neonatal cold injury. Much of my quarrel with the author appears to be with his choice of descriptive terms for the conditions he describes. However, it is this ambiguity that has led to most of the confusion in the literature. Such confusion will persist

NEONATAL SKIN DISORDERS

<i>To the Editor</i>: It seems that Dr. Szalay is disturbed about the nomenclature used in describing the three disorders in my article. He agrees with Elliot that the term subcutaneous fat necrosis seems adequate but that "preagonal induration" be substituted for sclerema neonatorum and "neonatal cold injury" for scleredema. Elliot suggested abandoning the term "sclerema" altogether. Szalay would like to use "sclerema" to encompass all three conditions. How this will contribute materially to the subject is hard to envisage. Certainly, more pediatricians, dermatologists, and pathologists are acquainted with the present nomenclature than with the terms suggested by Elliot and the correspondent. That "preagonal induration" and "neonatal cold injury" are more descriptive of the disease processes of sclerema neonatorum and scleredema, respectively, is debatable. EDITOR'S COMMENT: We conclude that until we know for sure what causes each of these disorders (1) terminology will necessarily be descriptive, and (2) we'd all better

Coexisting Heart Disease, Sclerema Neonatorum, and Possible Protozoan Infection

IN 1955, Barr reported the first case of interstitial plasmacellular pneumonia to be described outside of the European continent. Barr considered this condition to be of protozoan origin, and he based his opinion on the work of Vanek and Jirovec. Since Barr's paper was published, several instances of this disease have been reported in the American literature. Report of a Case From the histologie characteristics, the infant in our case apparently had a protozoan disease. The disease, rather than being localized to the lungs, was generalized, and sclerema neonatorum and congenital heart disease were associated. Congenital anomalies have been described in association with this disease; however, a coexisting sclerema neonatorum has not been mentioned. Clinical History .—After an apparently normal and spontaneous birth, a premature boy was placed in an incubator. The period of gestation was estimated to be 40 weeks. At the time of birth, the baby weighed 4 lb.

Subcutaneous adipose derangements of the newborn.

Confusion exists concerning the terminology and differentiation of the subcutaneous adipose diseases of the neonate. Of these uncommon disorders, subcutaneous fat necrosis (adiponecrosis subcutanea neonatorum) and sclerema neonatorum (sclerema adiposum) are encountered more often than scleredema (sclerema edematosum). Although scleredema probably represents an edematous form of sclerema neonatorum, some workers consider it a distinct clinical entity. <h3>Etiologic Aspects</h3> Since the causes of subcutaneous fat derangements of the newborn are unknown, one may rationally consider if a relationship exists between them, the connective tissue disorders, and reticuloendothelial diseases. The specificity of adipose tissue is a debatable question. Flemming, in 1870, stated that adipose tissue is ordinary connective tissue in which fat has been deposited. This contention was difficult to accept because some sites functioned as fat deposits, while other connective tissues were devoid of fat accumulations. Wells¹speculated whether at times connective tissue undergoes a transformation into adipose tissue, or

Sclerema neonatorum complicating surgical procedures.

In the past year, 3 infants from a single hospital service developed a condition following the description of sclerema neonatorum. Sclerema appeared to be a major factor in the deaths of two of the infants. Because so little attention has been paid this process in surgical literature, it seems pertinent to report the experience with these 3 patients and to review the more recent literature. Report of Cases Case 1. —A white male infant was brought to the Hospital at 3 days of age. Details of birth and past history were not available. Physical examination on admission revealed a 4 lb. 6½ oz. (2 kg.) infant with a rectal temperature of 95 F. The baby was cyanotic and moderately dehydrated and appeared acutely ill. There was obvious respiratory distress, with coarse rales and rhonchi audible throughout the chest. Subcutaneous tissue over the buttocks and lower extremities was cold, firm, and

Sclerema neonatorum treated with corticosteroids.

Sclerema neonatorum treated with corticosteroids.

Sclerema neonatorum treated with corticotropin (ACTH) report of two cases.

Sclerema neonatorum 1 is a peculiar induration of the skin and subcutaneous tissue that often starts in the buttocks and thighs and involves the remainder of the legs, the trunk, the arms, and the face. The soles, the palms, and the genitalia are spared. It is found in premature infants or in very young infants suffering from congenital debility, diarrheal conditions, dehydration, and circulatory stasis. There is associated marked lowering of body temperature; the skin is stiff, cold, and often mottled as in peripheral circulatory failure; and there is interference with activity, respiration, and sucking. When it appears, it is of very serious prognostic importance despite the fact that these infants have been treated for the primary disturbances such as dehydration, shock, and acidosis. Until recently, about 75% of the infants treated died. Reports of the successful treatment of sclerema neonatorum with corticotropin (ACTH) and cortisone in addition to the

Sclerema neonatorum; report of a case treated with ACTH.

SummaryA case of sclerema neonatorum in a 4‐week‐old boy, treated with ACTH with good effect, is reported.

Sclerema neonatorum successfully treated with corticotropin (ACTH).

SCLEREMA neonatorum is a diffuse hardening of the subcutaneous tissues occurring in undernourished, debilitated full-term and premature infants. 1 In an excellent review of the literature Hughes and Hammond 2 have summarized the significant findings of this condition as follows: (1) Average age of onset was 4 days with extremes from birth to 70 days; (2) 25 per cent of the mothers were ill at the time of delivery; (3) all but two deliveries were spontaneous; (4) average birth weight was 2,800 Gm. with variations from 2,150 to 4,100 Gm.; (5) the majority of infants exhibited abnormal behavior at birth, weakness and cyanosis being the most common symptoms; (6) almost all children had difficulty with body temperature control and evidences of other complications besides sclerema; (7) 75 per cent died, with the average age of death 12 days; (8) eleven infants were autopsied, thickening of connective tissue bands being the

Cortisone in the treatment of sclerema neonatorum.

SCLEREMA neonatorum is a condition characterized by induration of the skin and subcutaneous tissue and by local and general circulatory disturbances.1Recovery is extremely rare. In the following case the patient was treated with cortisone with apparent success. REPORT OF A CASE C. J., a 3 day old white boy, was admitted to the Medical College of Virginia Hospital on Oct. 21, 1950 with the diagnosis of sclerema neonatorum. The baby was born prematurely, the product of approximately 32 weeks' gestation. Labor was of three hours' duration, and delivery was uncomplicated. On the second day of life the local doctor noted that the infant's skin was abnormally indurated, and on the following day he referred the baby to the Medical College of Virginia Hospital for treatment. Physical examination at the time of admission revealed an apathetic 3 pound, 12 ounce (1,700 Gm.) white boy with a weak, feeble cry.

SCLEREMA NEONATORUM

Sclerema neonatorum is a rare disease occurring at or shortly after birth, characterized by a spreading induration of the skin and subcutaneous tissue from the lower extremities to the trunk, arms and face. The skin is reddish, purplish or mottled, cold, hard and leathery, while the subcutaneous fat becomes hard and rigid. It causes joints to be immobile, rendering impossible movements of the jaw, of the extremities and even of the thorax. Respirations become weak; the pulse is weak, and the temperature stays subnormal. Babies suffering from sclerema neonatorum are usually small and undernourished and are often premature. They almost invariably die within a few days after the onset of the disease. Sclerema neonatorum is thus sharply differentiated clinically from the disease that has been called adiponecrosis subcutanea neonatorum or lipophagic granuloma. This condition is benign; it appears a few weeks after birth and invariably disappears spontaneously, usually after a

Study of the Serum Lipids in Sclerema Neonatorum

ConclusionsAnalyses of the subcutaneous tissues in a 3 1/2-month-old, breast-fed female infant with sclerema neonatorum revealed the usual histologic and chemical alterations: chronic inflammatory reaction and presence of excess saturated fat. When the condition was improved repeat analyses revealed normal adipose tissue. During the course of the disease studies of the blood lipids were made. Early in the disease an increase in all the fractions of the blood lipids as well as a decrease in the iodine number, especially of the neutral fat, was found. When all signs of the disease had disappeared the blood lipids were normal. The lipemia is briefly discussed in the light of the alterations of the fat tissues, and it is suggested that a disturbance in fat deposition or in fat transport from the pathologic areas may be present in this disease. The clinical observation gives support to the idea that this tissue was meta-bolically altered.

SCLEREDEMA ADULTORUM (BUSCHKE)

In May 1900 Buschke 1 presented to the Berlin Dermatological Society a man aged 44 in whom a peculiar hardness of the skin had developed after an attack of influenza. The process began on the neck and spread rapidly upward to the face and down the chest and back. Buschke suspected that it was acute scleroderma, but he noted that the involvement seemed much deeper than the usual scleroderma. In the discussion of the case various opinions were expressed: Senator suggested a diagnosis of myxedema; Lesser was reminded of a process similar to sclerema neonatorum, which, however, had not been observed in adults; Blaschko stated that he had seen two patients with the identical condition, which appeared in one patient after scarlet fever and in the other after exposure to cold. Buschke 2 presented a second example of the disease to the same society in 1901, and first formally discussed

SCLEREMA NEONATORUM (SUBCUTANEOUS FAT NECROSIS)

There are two conditions in young infants which may properly be called sclerema. In early usage the term was probably employed to designate a diffuse hardening of the subcutaneous tissues occurring in weakly or marasmic infants, not necessarily new-born. It is a terminal condition, somewhat rare, sometimes seen as a complication in serious infantile diseases. For this entity the term preagonic induration has been suggested. The second condition, to which the term sclerema neonatorum may properly be applied, becomes evident within the first few weeks of life and usually disappears after three or four months. The lesions consist of sharply defined areas of induration, attached to the skin but not to the deeper tissues and affecting the back, the buttocks, the shoulders, the cheeks and the calves of the legs. The skin is frequently livid. Softening and fluctuation have been observed and occasionally formation of cysts. The general health is

ON THE IDENTITY OF ADIPONECROSIS SUBCUTANEA NEONATORUM WITH SCLEREMA NEONATORUM.

British Journal of DermatologyVolume 45, Issue 12 p. 498-506 ON THE IDENTITY OF ADIPONECROSIS SUBCUTANEA NEONATORUM WITH SCLEREMA NEONATORUM.* A. M. H. GRAY, A. M. H. GRAYSearch for more papers by this author A. M. H. GRAY, A. M. H. GRAYSearch for more papers by this author First published: December 1933 https://doi.org/10.1111/j.1365-2133.1933.tb10131.xCitations: 86 * Paper read at the Thirteenth Annual Meeting of the British Association of Dermatology and Syphilology, London, July 8th, 1933. Read the full textAboutPDF ToolsRequest permissionExport citationAdd to favoritesTrack citation ShareShare Give accessShare full text accessShare full-text accessPlease review our Terms and Conditions of Use and check box below to share full-text version of article.I have read and accept the Wiley Online Library Terms and Conditions of UseShareable LinkUse the link below to share a full-text version of this article with your friends and colleagues. Learn more.Copy URL Share a linkShare onFacebookTwitterLinked InRedditWechat Citing Literature Volume45, Issue12December 1933Pages 498-506 RelatedInformation

SUBCUTANEOUS FAT NECROSIS OF THE NEW-BORN

The various types of subcutaneous induration occurring in new-born or young infants have been the source of a good deal of confusion. During the past decade, however, knowledge of the subject has been considerably increased by histologic and chemical examinations. The type of induration that is the subject of this paper should. I think, be termed subcutaneous fat necrosis of the new-born, though it is still spoken of as sclerema neonatorum by some British and American writers. In many of these cases at the present time the confusion is merely one of nomenclature. Much confusion has also resulted from the reports of cases of scleroderma neonatorum which were manifestly not scleroderma but in all probability cases of subcutaneous fat necrosis. I shall discuss this subject later. The four principal types of subcutaneous induration to be considered are the following: adiponecrosis subcutanea neonatorum, sclerema adiposum (sclerema neonatorum),

SUBCUTANEOUS FAT NECROSIS

Subcutaneous fat necrosis is also called by the following terms: ischemic fat necrosis, traumatic fat necrosis of the new-born, pseudo-scleroderma, sclerema neonatorum, scleroderma neonatorum, curable induration of the skin of the new-born, adiponecrosis subcutanea neonatorum, necrosis adiposis neonatorum, erysipelitis infantum, Verhartung des Zellgewebes, Subcutane Fettgewebenecrosen beim neugeborenen, fettsklerose im sauglingsalter, Hautsklerom, scleroma dei neonati, sclereme des nouveau-nes, endurcissement du tissu cellulaire, Induration cutanee curable des nouveau-nes, cytosteatonecrose du tissu sous-cutane, sclereme adipeux en plaques. Under this varied nomenclature is described a condition of the skin in the new-born which is manifested by an area of pink or pinkish-blue discoloration, variable in size and circumference. The edges, sharply defined, may be slightly raised or flat, and the consistency is not unlike hard, cold dough, or as has been described, like the dead half frozen foetus. The skin does not pit on pressure. When grasped enmasse, the skin and the subcutaneous tissue